Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 7  |  Issue : 1  |  Page : 59-62

The impact of patient centered communication in managing Gardner's syndrome


1 Department of Diagnostic Sciences, Rutgers School of Dental Medicine, Newark, NJ, USA
2 Department of PGY-1, PG Periodontics, Stony Brook School of Dental Medicine, Stony Brook, NJ, USA

Date of Web Publication20-May-2015

Correspondence Address:
Dr. Steven R Singer
Room D-860, Rutgers School of Dental Medicine, 110 Bergen Street, Newark, NJ 07101-1709
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-8844.157401

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  Abstract 

Effective patient communication and comprehension are fundamental toward empowering the patient to make optimal health decisions. Barriers in patient health literacy extend beyond cultural and language differences and can significantly impede this process. This case report illustrates a major communication gap that resulted in contradictory perceptions between a treating oncologist and a patient. The patient's dentist was able to resolve this miscommunication and facilitate the patient's acceptance of the recommended intravenous chemotherapy for management of malignant desmoid tumors occurring secondary to Gardner's syndrome (GS). This report also documents classic craniofacial manifestations of GS including multiple unerupted supernumerary teeth, compound odontomas associated with a dentigerous cyst, as well as multiple osteomas in both arches and in the ethmoid and irregularly shaped radioopacities in both arches. In summary, effective patient-centered communication is a prerequisite for the optimal delivery of healthcare. Both interdisciplinary care and one-on-one patient-provider relationship center on coherent bidirectional communication.

Keywords: Gardner′s syndrome, interdisciplinary care, patient-centered communication


How to cite this article:
Subramanian G, Robles-Salas V, Quek SY, Singer SR. The impact of patient centered communication in managing Gardner's syndrome. J Orofac Sci 2015;7:59-62

How to cite this URL:
Subramanian G, Robles-Salas V, Quek SY, Singer SR. The impact of patient centered communication in managing Gardner's syndrome. J Orofac Sci [serial online] 2015 [cited 2019 Jul 19];7:59-62. Available from: http://www.jofs.in/text.asp?2015/7/1/59/157401


  Introduction Top


Communication in healthcare must effectively transcend cultural, language and patient health literacy barriers in order to empower the patient in making optimal health decisions. This case report illustrates a serious communication gap that resulted from failure of the clinician to ensure patient comprehension. The fact that the presence of a language interpreter during the doctor-patient interaction was not enough to enable patient comprehension strongly suggests that the communication barrier extended beyond language differences. This case report also illustrates classic radiographic craniofacial manifestations of Gardner's Syndrome.


  Case Report Top


Chief complaint

A 30-year-old Hispanic male patient presented to the emergency clinic at our dental school upon referral from his physician. He was told to get "dental clearance" in anticipation of chemotherapy for "abdominal tumors." The patient, who was Spanish-speaking, also reported discomfort in the mandibular left premolar area. It should be noted that the patient presented without a written consultation request, name of the referee, history or proposed chemotherapeutic treatment, or diagnosis.

The patient's presentation to medical oncology, obtained later, included a chief complaint of multiple growths in the abdomen with a history of resection. His past medical history included a diagnosis of Gardner's syndrome (GS), intestinal polyposis, desmoid tumors, and sphenoethmoid osteoma (causing seizures). The patient's family history reveals that his father and sister were lost to malignant intestinal disease.

Dental examination revealed several missing teeth, restorations, and inflamed gingiva. Tooth number 20 was fractured. A panoramic radiograph was prescribed and exposed [Figure 1]. The panoramic radiograph revealed multiple impacted supernumerary teeth, compound odontomas with associated dentigerous cysts, and osteomas. From the patient's medical history and the radiographic appearance of the jaws, these changes were diagnosed as signs of GS in the craniofacial region. In addition, the panoramic radiograph revealed the presence of residual roots and nonrestorable caries in teeth number's 20, 28, 29, as well as horizontal and vertical bone loss suggestive of marginal periodontitis. A cone-beam computed tomography (CBCT) examination was prescribed to facilitate treatment planning for removal of the dentigerous cyst and compound odontoma in the right anterior maxilla. In addition to providing multiplanar projections of the lesions in the mandible and maxilla for treatment planning, the scan further revealed the presence of an osteoma in the left anterior ethmoid air cells [Figure 2] and [Figure 3].
Figure 1: Panoramic projection demonstrating bilateral expansion of the alveolus into the maxillary air sinus


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Figure 2: (a) Axial projection: Cystic lesions surrounding impacted teeth seen. (b) Right maximum intensity projection lateral view: Impacted supernumerary teeth and odontomas. (c) Axial projection: Osteoma in left ethmoid air cells


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Figure 3: (a) Oblique view demonstrating multiple supernumerary, odontomas and cysts in the maxilla. (b) Left maximum intensity projection view demonstrating compound odontomas. (c) Oblique view demonstrating expansile altered bone displacing the floor of the maxillary air sinus


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Dental management included extraction of mandibular premolar teeth number's 20, 28, 29 and follow-up to monitor initial healing. Invasive craniofacial corrective and resective surgery was delayed due to the patient's need to begin chemotherapy.

Further conversation with the patient's oncologist revealed that the patient's earlier discussions with the physician took place with a translator present, as the patient spoke only Spanish and the physicians were not able to speak Spanish. The patient had agreed only to start "oral chemotherapy" (tamoxifen) and was reluctant to start intravenous (IV) chemotherapy. It was the oncologist's perception that the patient was in denial and so, was reluctant to start IV chemotherapy. During the dental visit, since the dental resident managing the case (V R-S) was bilingual Spanish and English, she quickly learned that the patient's perception differed from that of the oncologist. The patient thought that there was a choice and so chose to take "chemotherapy" orally rather than as IV medication. Even worse, the patient was unaware of his poor prognosis and the futility of multiple surgeries.

Once the diagnosis, prognosis and treatment options were clarified with the patient, he immediately agreed to start IV chemotherapy without further delay. The dental resident communicated this with the oncology team responsible for managing the patient. The patient expressed his gratitude and committed to follow through with his treatment recommendations.


  Discussion Top


This case clearly illustrates a serious communication gap that was attributable to various factors that included limited health literacy, cultural and linguistic barriers, and multiple centers of care with limited communication between centers. Racial and ethnic disparities in healthcare undermine optimal delivery of critical healthcare services and the importance of culturally competent healthcare in overcoming these barriers cannot be overstated. [1] Oral health disparities are more extensively documented in the United States than elsewhere, however, these disparities are probably not unique to just the western hemisphere. [2],[3]

Effective communication must include gathering information, communication at the level of patient's comprehension, and communication across health centers and teams. Empowerment of the patient to make informed healthcare decisions relies on multiple factors, including provider competence, trustworthiness, cultural competence, communication with patients and families and information quality. [4] In fact, national standards for culturally and linguistically appropriate healthcare services have been identified. [5]

In the case scenario described above, having a translator alone was not adequate to facilitate a coherent discussion during a crucial juncture in the patient's oncological treatment. The barrier to such a dialog extends beyond language differences and the case described above highlights the need for a broader understanding. Such miscommunications may occur during the dental assessment and management of patients as well.

This manuscript also illustrates several classic craniofacial manifestations of GS. GS was originally described in the year 1951 by Eldon J Gardner, as characterized by diffuse intestinal polyposis, osteomas, fibromas and cysts that appeared to have an autosomal dominant pattern of inheritance, clustering in a family. [6] Subsequently, the association with mutations in the adenomatous polyposis coli (APC) gene underscored the similarities of GS to familial adenomatous polyposis (FAP). Still the extra intestinal manifestations such as the osteomas and skin tumors as well as soft-tissue tumors are distinct features of GS (reviewed in). [7] GS follows an autosomal dominant inheritance pattern with almost complete penetrance and variable expressivity. [8]

GS constitutes nearly 1% of all colorectal cancers and has an incidence of about 1:10,000. [9] This condition, characterized by the presence of numerous tubulovillous adenomas in the colon, results in colorectal cancer by the fourth decade of life, if untreated. FAP is an autosomal dominant disorder and the diagnosis of FAP should be followed by a careful evaluation of family members, as such evaluation may allow for early diagnosis and optimal management. [10] Molecular genetic testing of APC detects pathogenic variants in up to 90% of individuals with typical FAP. [11]

Peripheral or central osteomas are a hallmark for GS and are essential for its diagnosis. While most often asymptomatic, they often cause disfigurement, especially when they occur in the skull, mandible, facial bones or the paranasal air sinuses. Common oral and maxillofacial manifestations of GS include jaw osteomas, odontomas and supernumerary or impacted/ unerupted teeth. The use of CBCT has been demonstrated to be a valuable aid in the diagnosis and assessment of dental and craniofacial involvement. [10] The osteomas demonstrate increased uptake with functional imaging such as single-photon emission computed tomography (SPECT/CT), indicative of increased bone remodeling activity, while displaying no uptake on fluoro-deoxyglucose-positron emission tomography, consistent with their developmental, rather than inflammatory origin. [12],[13]

As oral signs usually precede gastrointestinal symptoms, the dentist may greatly facilitate early diagnosis of GS and timely referral of this life-threatening condition. [14] A high incidence of colorectal cancer and the occurrence of massive abdominal desmoid tumors are the two chief reasons for mortality in GS. Treatment options for desmoid tumors include antioestrogens, chemotherapy, surgery, or radiotherapy, as were offered to the patient described in this case report. While benign, surgical management of craniofacial manifestations of GS is sometimes necessitated by the patient's esthetic and functional needs. [8],[15],[16]


  Conclusion Top


This case report presented an ideal forum to illustrate classic craniofacial manifestations of GS as well as highlight the importance of effective interdisciplinary and patient-provider communication in the optimal delivery of healthcare.

 
  References Top

1.
Brach C, Fraser I. Reducing disparities through culturally competent health care: An analysis of the business case. Qual Manag Health Care 2002;10:15-28.  Back to cited text no. 1
    
2.
Kressin NR. Racial/Ethnic disparities in health care: Lessons from medicine for dentistry. J Dent Educ 2005;69:998-1002.  Back to cited text no. 2
    
3.
Chattopadhyay A. Oral health disparities in the United States. Dent Clin North Am 2008;52:297-318, vi.  Back to cited text no. 3
    
4.
Dy SM, Purnell TS. Key concepts relevant to quality of complex and shared decision-making in health care: A literature review. Soc Sci Med 2012;74:582-7.  Back to cited text no. 4
    
5.
U.S. Department of Health and Human Services, O.o.M.H.H.O., National standards for Culturally and Linguistically Appropriate Services (CLAS) in health and health care: A blueprint for advancing and sustaining CLAS Policy and Practice., O.o.M.H.H.O. U.S. Department of Health and Human Services, Editor. 2012. Washington D.C., USA.  Back to cited text no. 5
    
6.
Gardner EJ. Follow-up study of a family group exhibiting dominant inheritance for a syndrome including intestinal polyps, osteomas, fibromas and epidermal cysts. Am J Hum Genet 1962; 14:376-90.  Back to cited text no. 6
    
7.
Gómez García EB, Knoers NV. Gardner's syndrome (familial adenomatous polyposis): A cilia-related disorder. Lancet Oncol 2009;10:727-35.  Back to cited text no. 7
    
8.
Cristofaro MG, Giudice A, Amantea M, Riccelli U, Giudice M. Gardner's syndrome: A clinical and genetic study of a family. Oral Surg Oral Med Oral Pathol Oral Radiol 2013;115:e1-6.  Back to cited text no. 8
    
9.
Cole TR, Sleightholme HV. ABC of colorectal cancer. The role of clinical genetics in management. Br Med J 2000;321:943-6.  Back to cited text no. 9
    
10.
Kamel SG, Kau CH, Wong ME, Kennedy JW, English JD. The role of Cone beam CT in the evaluation and management of a family with Gardner's syndrome. J Craniomaxillofac Surg 2009;37:461-8.  Back to cited text no. 10
    
11.
Jasperson KW, Burt RW. APC-associated Polyposis Conditions., in GeneReviews. 1993-2015. Seattle (WA): University of Washington, Seattle; 1993-2015.  Back to cited text no. 11
    
12.
Sohn MH, Jeong YJ, Lim ST, Kim DW, Jeong HJ, Yim CY. F-18 FDG PET/CT Findings of Spontaneous Mesenteric Fibromatosis in a Patient with Gardner's Syndrome. Nucl Med Mol Imaging 2011;45:156-7.  Back to cited text no. 12
    
13.
Kim WH, Kim DW, Kim CG, Kim MH. Additional Detection of Multiple Osteomas in a Patient with Gardner's Syndrome by Bone SPECT/CT. Nucl Med Mol Imaging 2013;47:297-8.  Back to cited text no. 13
    
14.
Wijn MA, Keller JJ, Giardiello FM, Brand HS. Oral and maxillofacial manifestations of familial adenomatous polyposis. Oral Dis 2007;13:360-5.  Back to cited text no. 14
    
15.
Boffano P, Bosco GF, Gerbino G. The surgical management of oral and maxillofacial manifestations of Gardner syndrome. J Oral Maxillofac Surg 2010;68:2549-54.  Back to cited text no. 15
    
16.
Panjwani S, Bagewadi A, Keluskar V, Arora S. Gardner's Syndrome. J Clin Imaging Sci 2011;1:65.  Back to cited text no. 16
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